Continuous Positive Airway Pressure plus Mandibular Advancement Therapy (PAPMAT): study protocol for an adaptive randomised crossover trial comparing the benefits and costs of combining two established treatments for obstructive sleep apnoea.

Background: Obstructive sleep apnoea (OSA) involves repeated breathing pauses during sleep due to upper airway obstruction. It causes excessive daytime sleepiness and has other health impacts. Continuous positive airway pressure (CPAP) therapy is effective first line treatment for moderate to severe OSA.

Rare PSAP Variants and Possible Interaction with GBA in REM Sleep Behavior Disorder.

Background: PSAP encodes saposin C, the co-activator of glucocerebrosidase, encoded by GBA. GBA mutations are associated with idiopathic/isolated REM sleep behavior disorder (iRBD), a prodromal stage of synucleinopathy.

Objective: To examine the role of PSAP mutations in iRBD.

Genotype, extrapyramidal features, and severity of variant ataxia-telangiectasia.

Objective: Variant ataxia-telangiectasia is caused by mutations that allow some retained ataxia telangiectasia-mutated (ATM) kinase activity. Here, we describe the clinical features of the largest established cohort of individuals with variant ataxia-telangiectasia and explore genotype-phenotype correlations.

Methods: Cross-sectional data were collected retrospectively.

Effect of Home Noninvasive Ventilation With Oxygen Therapy vs Oxygen Therapy Alone on Hospital Readmission or Death After an Acute COPD Exacerbation: A Randomized Clinical Trial.

Importance: Outcomes after exacerbations of chronic obstructive pulmonary disease (COPD) requiring acute noninvasive ventilation (NIV) are poor and there are few treatments to prevent hospital readmission and death.

Objective: To investigate the effect of home NIV plus oxygen on time to readmission or death in patients with persistent hypercapnia after an acute COPD exacerbation.

Design, Setting, And Participants: A randomized clinical trial of patients with persistent hypercapnia (Paco2 >53 mm Hg) 2 weeks to 4 weeks after resolution of respiratory acidemia, who were recruited from 13 UK centers between 2010 and 2015.

The impact of changing people with sleep apnea using CPAP less than 4 h per night to a Bi-level device.

Pressure intolerance is a reason for poor acceptance and subsequent compliance in some patients starting treatment with continuous positive airway pressure (CPAP). In unselected populations initiating CPAP; different types of pressure generating device have not been found to improve compliance. We hypothesized that using Bi-level PAP for patients who reported pressure related discomfort as a cause for poor compliance with CPAP might increase their hours of treatment use.

Volume assured versus pressure preset non-invasive ventilation for compensated ventilatory failure in COPD.

Background: The addition of domiciliary non-invasive ventilation (NIV) to standard therapy in chronic obstructive pulmonary disease (COPD) patients with compensated ventilatory failure (CVF) is reported to have beneficial effects. Compliance with NIV is an important factor. Volume assured NIV (va-NIV) may improve compliance and ventilation during sleep by automatically titrating ventilatory pressures.

A crossover randomised controlled trial of oral mandibular advancement devices for obstructive sleep apnoea-hypopnoea (TOMADO).

Rationale: Mandibular advancement devices (MADs) are used to treat obstructive sleep apnoea-hypopnoea syndrome (OSAHS) but evidence is lacking regarding their clinical and cost-effectiveness in less severe disease.

Objectives: To compare clinical- and cost-effectiveness of a range of MADs against no treatment in mild to moderate OSAHS.

Measurements And Methods: This open-label, randomised, controlled, crossover trial was undertaken at a UK sleep centre.

A randomised crossover trial comparing volume assured and pressure preset noninvasive ventilation in stable hypercapnic COPD.

Recent randomised controlled trials suggest non-invasive ventilation may offer benefit in the long-term management of ventilatory failure in stable COPD. The best mode of ventilation is unknown and newer volume assured modes may offer advantages by optimising ventilation overnight when treatment is delivered. This study compares volume assured with pressure preset non-invasive ventilation.

Is there an alternative to pre-flight hypoxic challenge testing in scoliotic patients?

Introduction: Hypoxic challenge testing (HCT) is not readily available in all hospitals. It has recently been shown that resting oximetry does not reliably predict the results of HCT in patients with extrapulmonary restrictive lung disease. We assessed other clinical tests to see if they might be used as an alternative screen for HCT.

Long-term non-invasive ventilation to manage persistent ventilatory failure after COPD exacerbation.

Background And Objective: Patients with ventilatory failure at discharge from hospital following an exacerbation of COPD (ECOPD) have increased work of breathing and reduced inspiratory muscle strength compared with those with a normal arterial carbon dioxide tension (PaCO(2)). They also have a significantly worse prognosis. Long-term non-invasive positive pressure ventilation (NIPPV) may offer a treatment strategy but benefits have not been established.

The effects of withdrawing long-term nocturnal non-invasive ventilation in COPD patients.

Patients with ventilatory failure due to chronic obstructive pulmonary disease (COPD) are increasingly managed with long-term non-invasive positive pressure ventilation (NIPPV) and this may improve survival. NIPPV can frequently be interrupted but there are few data detailing the short-term effects and none on the longer-term consequences of treatment withdrawal. Ten patients withdrew from NIPPV for 1 week and were randomised to restart NIPPV or to continued withdrawal for up to 6 months.

Weaning from prolonged invasive ventilation in motor neuron disease: analysis of outcomes and survival.

Introduction: Non-invasive ventilation (NIV) improves prognosis in patients with motor neuron disease (MND) in the absence of major bulbar involvement. However, some experience a rapid and unexpected decline in respiratory function and may undergo emergency tracheal intubation. Weaning from invasive ventilation can be difficult, and reported independence from invasive ventilation is uncommon with poor prognosis.

A bench test to confirm the core features of volume-assured non-invasive ventilation.

Unlabelled: Volume-assured non-invasive ventilation (NIV) theoretically guarantees minute ventilation with circuit leak compensation unlike other modes of NIV. Bench testing demonstrated that minute ventilation was maintained with varying lung compliance and resistance with minimal effect from circuit leak, confirming for the first time the core features of volume-assured NIV.

Background And Objective: Volume-assured non-invasive positive pressure ventilation (va-NIPPV) is a novel mode designed to adapt pressure support (PS) to achieve a target minute ventilation (TgV).